ABSTRACT
Congenital renal malformations are the most common congenital malformations in humans, the most common being horseshoe kidney with joined lower poles present in up to 0.25% of general population. To the contrary renal fusions with ectopia are amongst the rare malformations affecting 1 in 2000 examined cadavers. Males are affected slightly more often then females (3:2), left to right crosses being more frequent. The value of early diagnosis of asymptomatic renal malformation is uncertain, except when other abnormalities might point to them and they can affect the clinical management strategy (severe ear deformity with facial malformation, gynecological abnormalities). In females concomitant abnormalities of kidneys and paramesonephric duct derivatives are common, with up to 40% of women with uterine malformations reported to have various congenital renal abnormalities. Even though there are occasional examples of surgical repair of the injured kidneys with crossed-fused malformation the majority of surgical cases resort to renal resection. We presented a rare case of complex genitourinary malformation with crossed fused disk kidney diagnosed after the iatrogenic injury to the right ureter after emergent gynecological surgery. Rare pathology and presentation was diagnosed by IVP and confirmed during operation. We have constructed an ipsilateral ureteroureterostomy to drain the right portion of fused kidney. Urinary flow was satisfactory with no significant nephrostomy tube drainage next day, allowing the nephrostomy tube removal. Postsurgical evaluation by IVP has demonstrated a good flow from both kidney and no detectable anastomotic leaks. Postsurgical recovery was uneventful. After 2 years from operation IVP data did not changed. It must be noted, that Ectopic kidneys are frequently malformed and have an abnormal ureteral drainage leading to incontinence and frequent UTIs.
